A 63-year-old male was admitted to our hospital because of fever up, systemic lymphadenopathy and skin eruption. On admission, a peripheral blood examination showed a hemoglobin concentration of 10.1g/dl, a reticulocyte ratio of 42‰, an undetectable haptoglobin concentration below 10ng/dl, LDH of 558IU/l and strong positive results of direct/indirect Coombs test showed the existence of auto-antibodies. These data indicated autoimmune hemolytic anemia (AIHA). The skin biopsy was done and revealed an allergic dermatitis, since systemic skin eruption was accompanied by IgE concentration of 14300IU/ml and eosinophilia (14%). Immunoblastic lymphadenopathy-like T cell lymphoma was diagnosed on the lymph node biopsy. Biweekly CHOP-E therapy obtained improvement of skin eruption, lymphadenopathy, AIHA and hypergammaglobulinemia (including IgE). These findings suggest that T type lymphoma cells produced high concentration (652pg/ml) of IL-2, enhancing immune reactions and activating B cell functions, such as an allergic dermatitis and AIHA.

View full abstract

We have experienced a case of chronic active Epstein-Barr virus infection (CAEBV) complicated in systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS). A 35-year-old woman was admitted to our hospital with complaints of fever and dyspnea on exertion. She was diagnosed as having SLE on the basis of arthritis, oropharyngeal ulcer, lymphopenia, and positive autoantibodies against DNA, RNP and SS-A. The diagnosis of APS was also made because of positive anti-cardiolipin IgG antibodies and the existence of multiple pulmonary infarction with pulmonary hypertension. The administration of 30mg/day of prednisolone and anti-coagulation significantly improved clinical symptoms. However, she was again admitted to the hospital four months later because of progressive liver damage and pancytopenia. Increment of prednisolone did not improve the clinical situation and she expired because of pulmonary hemorrhage.
At autopsy, there were a significant increase of histiocytes with hemophagocytosis and a dense infiltration of atypical lymphocytes in the liver, spleen, lymph nodes and bone marrow. Infiltrated lymphocytes were positive for CD 3 and EBER 1 in immunohistochemical staining and EBVmRNA was detected by in situ hybridization. Final pathological diagnosis was CAEBV with hemophagocytic syndrome in association with lupus nephritis, pulmonary hemorrhage and pulmonary infarction.

View full abstract

Since July, 1999, a 66 year-old man had been complaining of dry cough. He noticed submandibular swelling, lacrimal gland enlargement and dry eye. Keratoconjuctivitis sicca was detected by an ophthalmologist.
Sjögren's syndrome was diagnosed based on microscopic findings of a labial salivary gland biopsy although anti-SS-A and anti-SS-B antibodies were negative.
Hypergammmaglobulinemia (IgG 3916mg/dl) and IgA-M-proteinemia were pointed out. Swelling of mediastinal and abdominal lymph nodes was detected together with enlargement of salivary and lacrimal glands. We suspected the existence of malignant lymphoma, but a biopsy of lacrimal glands showed only lymphocytic and plasma cell infiltration and immuno-histochemical analysis denied monoclonality of lymphoid line.
An administration of corticosteroids caused rapid diminution in size of lacrimal and submandibular glands and lymph nodes. Clinical symptoms were also improved, but IgA-M proteinemia remains. The characteristics of our case were enlargement of lacrimal glands, the negativity of anti SS-A and SS-B antibodies, atypical onset and M-proteinemia. We discussed about these characteristics of Sjögren's syndrome in our case.

View full abstract

We report two patients with scleroderma, 73-year-old female and 67-year-old female, who developed anti neutrophil cytoplastic autoantibody (ANCA) associated rapid progressive glomerulonephritis (RPGN). Both patients have had a long history of scleroderma (23 and 14 years, respectively) when ANCA-associated glomerulonephritis occured.
In the first patient, scleroderma was localized in both fingers. She has been followed-up as CREST syndrome rather than systemic sclerosis. The complaints on admission were leg edema and left chest pain in the first patient, and a pyrexia and dyspnea in the second patient. Both patients showed pulmonary manifentation (pleural effusion in the first patient, interstitial pneumonia and alveolar hemorrhge in the second patient, respectively) and rapid progerssive glomeulonephritis. Both patients died in spite of corticosteroid therapy. Autopsy findings in the second patient demonstrated crescentic glomerulonephritis and alveolar hemorrhage.
Our cases demonstrated that MPO-ANCA associtated glomerulonephritis could be associated with limited scleroderma as well as systemic scleroderma. In these condition, the prognosis will be poor if scleroderma seemed to be stable.

View full abstract